Aim Eosinophilic fasciitis (EF) is a rare, fibrosing disorder of skin and subcutaneous tissue. This study was undertaken to describe its clinical and laboratory features and identify prognostic factors associated with outcome. Methods We conducted a retrospective review of all EF patients evaluated at our institution from 1 January1997 to 30 December 2016. Kaplan‐Meier methods were used to determine treatment response rates over time. Potential associations between baseline characteristics and complete response were examined using Cox models adjusted for age and sex. Time‐dependent covariates were used to examine treatment effects. Results We identified 89 EF patients, with a female‐to‐male ratio of 1:1. Clinical features included groove sign in 26 (29%), peau d'orange/dimpling in 22 (25%), inflammatory arthritis in 9 (10%) and muscle weakness in 9 (10%). Aldolase was elevated in 11/36 (31%). Complete response rate was 60% (95% confidence interval [CI]: 35‐75) at 3 years. Diagnostic delay was inversely associated with treatment response (hazards ratio: 0.84 per 1 month increase; 95% CI: 0.73‐0.98). No baseline characteristics correlated with treatment response, but a trend toward positive association of elevated aldolase, hypergammaglobulinemia and presence of hematologic disorders was noted. Methotrexate was the most commonly used immunosuppressant in 79%, hydroxychloroquine in 45%, mycophenolate mofetil in 18% and azathioprine in 8%. No single immunosuppressant agent was associated with a superior response during treatment. Conclusions EF is characterized by relatively high response rates. Consensus diagnostic criteria, standardized management algorithms, and large prospective multi‐center cohorts are needed to develop an evidence‐directed approach to this challenging condition.

Background:Visual ischemia (VI) is one of the most feared complications in giant cell arteritis (GCA). While the frequencies of VI development at or near diagnosis are better studied, limited information is available regarding the frequency of VI during relapse.Objectives:The purpose of this study was to characterize the frequency of visual ischemia (VI) as a manifestation of relapse or during follow-up in patients with GCA through performance of a systematic literature review.Methods:Potentially eligible studies were identified from Medline and EMBASE databases from inception to November 31, 2019 using a search strategy that comprised of terms for “giant cell arteritis,” “temporal arteritis,” or “Horton’s disease,” with “relapse,” “recurrence,” “flare,” “outcome,” “follow-up,” or “prognosis.” VI was defined as transient or permanent, full or partial, monocular or binocular visual field loss. VI occurring within 4 wks of GCA diagnosis was considered due to active disease and not included as a relapse event. Inclusion criteria used: (1) original research reported in English, (2) GCA definition provided, (3) VI outcome described as one of the following: (a) relapse rate/frequency denoting the presence or absence of VI, or (b) absolute number of VI events (> 4 weeks after GCA diagnosis) even if total cohort relapse rate/frequency was not provided. In order to reduce bias from under-reporting of negative results, studies that reported relapse rates/frequencies with accompanying relapse characteristics but did not provide initial detail regarding the presence/absence of VI were also identified. In such circumstances, the primary authors were directly contacted for patient-level data regarding VI and these studies were included in the final analysis if such data were available and provided.Results:A total of 913 unique articles were identified and underwent screening. Among these, 148 articles underwent independent full-text review by two physicians (K.B. and M.J.K). 33 articles met full inclusion criteria and an additional 21 articles included data on relapse but did not report VI patient data in the publication. Responses were received from authors of 11 of these 21 studies allowing for inclusion. 44 studies accounting for 3,649 patients with GCA were identified. Average percentage of baseline VI was 19% (range 0-66%). The average length of follow-up was 3.4 years (range 0.4 to 8.7). VI developing > 4 weeks after GCA diagnosis was recorded in a total of 53 patients (1.5%).Study-defined relapses were reported in 36 studies. A total of 1,215 patients with at least one or more relapses were recorded among 2,592 patients under observation (47%). Among these 36 studies, VI occurred in 37 patients (3.0%) with at least one study defined relapse event.Comparing trial design, retrospective studies (n=25) reported 27 of 2,718 (1%) patients developed VI during follow-up whereas 19 of 541 (3.5%) patients in randomized controlled trials (n=8) developed VI during the trial or post-trial follow-up.Conclusion:This report outlines the first systematic review evaluating VI as a manifestation of relapse and during follow-up in GCA. Overall, VI > 4 weeks after GCA diagnosis is uncommon (1.5%) but is noted in up to 3% of patients with at least one relapse event. Frequencies of reported VI were 3.5 times higher in randomized controlled trials compared to retrospective studies.Disclosure of Interests:Kubra Bugdayli: None declared, Patompong Ungprasert: None declared, Kenneth J Warrington Grant/research support from: Financial support for research from Kiniksa, Eli Lilly, Matthew Koster: None declared

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